Haematology Flashcards
Causes of microcytic anaemia
iron deficiency - diet, malabsorption or blood loss
thalassaemia
causes of normocytic anaemia
chronic disease
acute blood loss
renal failure - EPO deficient
RBC destruction
causes of macrocytic anaemia
B12 def
Folate def
pregnancy
alcohol
hypothyroidism
aplastic anaemia
myeloma
liver disease
what can cause haemolytic anaemias
note these are rare
- congenital; membrane, enzyme (G6PD) of Hb disorders of RBCs
- acquired; wilson’s disease, autoimmune, drugs
management of beta thal major
long term blood transfusions + iron chelation
tests for haemolysis
Coomb’s test:
1. indirect antiglobulin test - to cross match blood
2. Direct Antiglobulin Test - detect patients own antibodies to their red cells
mechanism of action and reversal of dabigatran
direct thrombin inhibitor
idarucizumab to reverse
universal FFP donor
AB RhD negative
what is lymphoma? how does it normally present
normal lymphoid structure replaced by malignant cells
bimodal ages; younger then in older age
Smooth, firm lymph nodes, dry cough, tiredness and B symptoms (weight loss, night sweats and fever)
types of lymphoma
hodgkins; reed-sternberg cells
non-hodgkins:
- diffuse large B cell (high grade)
- follicular NHL (low grade)
- T cell
lymphoma staging system
Ann Arbour
stage 1 - one group of nodes
stage 2 - 2 or more groups but one side of the diaphragm
stage 3 - both sides of the diaphragm
stage 4 - organ involvement (bone, liver, lungs, spleen)
A or B is also assigned based on whether B symptoms present
types of leukaemia
Myeloid - acute or chronic
Lymphocytic - acute or chronic
acute - ‘blasts’ on blood film. Pt is anaemic +/- thrombocytopenic. bone marrow failing
chronic - mature cells seen in excess numbers but are malignant and abnormal
what is multiple myeloma?
what symptoms and signs?
malignancy arising from the antibody producing plasma B cells
Calcium raised with normal phosphate and ALP
Renal impairment
Anaemia
Bone pain - often back pain. osteolytic lesions in bone
bence jones proteins in urine and plasma
what does prothrombin time measure? what factors affect it?
extrinsic pathway
factors, II, V, VII X
warfarin also affects it
what does activated partial thromboplastin time (APTT) measure? what factors/things affect it?
intrinsic pathway
factors VIII, IX, XI, XII
also heparin and von willebrand deficiency
what causes both PT and APTT to be prolonged?
vitamin K deficiency
disseminated intravascular coagulation
what is the premise of the 50:50 plasma test?
mix 50% patients plasma and 50% normal
if problem is a factor deficinecy then clotting times will correct
if an inhibitor is present then it wont correct
Haemophilia A and B
what are they?
management?
A = factor VIII deficiency
B = factor IX deficiency
A; give factor VIII 3x daily. amount depends on severity
also can give desmopressin
B; give factor IX 1x daily
both can give tranexamic acid in a bleed
CLL complications
- anaemia
- hypogammaglobulinaemia leading to recurrent infections
- warm autoimmune haemolytic anaemia in 10-15% of patients
- transformation to high-grade lymphoma (Richter’s transformation)
management of essential thrombocytosis
hydroxyurea
what is von willebrands disease?
symptoms
types
inherited bleeding disorder of reduced vWF which is a carrier for factor VIII and promotes platelet adhesion
epistaxis and menorrhagia are common symptoms
type 1 - partial reduction (80% of patients)
type 2 - abnormal vWF
type 3 - no vWF
investigation results in von willebrands disease
management
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation
management: Tranexamic acid for bleeds, desmopressin, factor VIII concentrate
causes and blood results of Disseminated Intravascular Coagulation
causes:
- trauma
- sepsis
- obstetric complications
- malignancy
blood results:
decreased platelets and fibrinogen
increased APTT, PT
long term management of sickle cell disease
hydroxyurea
pneumococcal vaccine every 5 years
management of a sickle cell crisis
analgesia e.g. opiates
rehydrate
oxygen
consider antibiotics if evidence of infection
blood transfusion
exchange transfusion: e.g. if neurological complications
blood transfusion complications
-immunological: acute haemolytic, non-haemolytic febrile, -allergic/anaphylaxis
-infective
-transfusion-related acute lung injury (TRALI)
-transfusion-associated circulatory overload (TACO)
-other: hyperkalaemia, iron overload, clotting
typical presentation of CML
60-70 years old
anaemia –> lethargy
drenching night sweats
massive splenomegaly
weight loss
leukocyte alkaline phosphatase
thrombocytosis and lymphocytosis
treatments of:
CML
CLL
NHL
CML = imatinib
CLL = Fludarabine, cyclophosphamide and rituximab (FCR). second line is ibrutinib
NHL = Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone (R-CHOP) is used in the treatment of Non-Hodgkin’s lymphoma (NHL).
ALL presentation
typically 2-5 years old
anaemia and splenomegaly
increased lymphocytes, + neutropenia and thrombocytopenia
what is the preferred anticoagulant if renal function is impaired?
apixaban
Iron profile in haemochromatosis
Raised transferrin and ferritin. Low total iron binding capacity
threshold for transfusion in anaemia
gernerally <70g/l
in ACS <80g/L
symptoms and treatment for immune thrombocytopenia
more common in females. presents with petechiae, epistaxis and bleeding gums
first line treatment is oral prednisolone
test for autoimmune haemotlyic anaemia
direct coombs test
polycythaemia vera
what is it
risks
treatment
prognosis
myeloproliferative disorder caused by clonal proliferation of a marrow stem cell where all cell counts are raised particularly RBCs
increased risk of thrombotic events - stroke and MI
patients should be on low dose aspirin
venesection to reduce Hb
chemotherapy can be beneficial
thrombotic events common
5-15% of patients progress to myelofibrosis
5-15% of patients progress to acute leukaemia
Which clotting factors are affected by heparin
Prevents activation of factors 2, 9, 10, 11
What clotting factors are affected by warfarin
2, 7, 9, 10
management of anti-phospholipid syndrome in pregnancy
low dose aspirin once pregnancy confirmed on urine testing
LMWH once fatal heart on ultrasound then discontinue at 34 weeks
general features of autoimmune haemolytic anaemia
Normocytic anaemia
Reticulocytosis
Raised LDH and bilirubin
management of autoimmune haemolytic anaemia
warm; steroids +/- rituximab
cold; responds less well to steroids
how does beta thal major present
in first year of life with failure to thrive and hepatosplenomegaly
what is desferrioxamine
iron chelation therapy
in what conditions are ‘target cells’ seen on blood film
Sickle-cell/thalassaemia
Iron-deficiency anaemia
Hyposplenism
Liver disease
in what conditions are ‘tear-drop’ poikilycytes seen on blood film
myelofibrosis
in what conditions are ‘spherocytes’ seen on blood film
Hereditary spherocytosis
Autoimmune hemolytic anaemia
in what conditions are ‘basophilic stippling’ seen on blood film
Lead poisoning
Thalassaemia
Sideroblastic anaemia
Myelodysplasia
in what conditions are Howell-Joly bodies seen on blood film
hyposplenism
in what conditions are Heinz bodies seen on blood film
G6PD deficiency
Alpha-thalassaemia
in what conditions are Schistocytes (‘helmet cells’) seen on blood film
Intravascular haemolysis
Mechanical heart valve
Disseminated intravascular coagulation
in what conditions are pencil poikilocytes seen on blood film
iron deficiency anaemia
in what conditions are Burr cells seen on blood film
Uraemia
Pyruvate kinase deficiency
types of blood transfusion reaction
Immunological
Infective
Transfusion Related Lung Injury
Transfusion Associated Circulatory Overload
Non Haemolytic febrile reaction to blood transfusion
Fever and chills
slow or stop the transfusion, paracetamol and monitor
minor allergic reaction to blood products
pruritus and urticaria
temporarily stop the transfusion, give antihistamines and monitor
anaphylaxis reaction to blood products
hypotension, dyspnoea, wheezing, angioedema
stop the transfusion, IM adrenaline and A-E support
Acute haemolytic reaction to blood products
ABO incompatibility - human error
Fever, abdominal pain, hypotension
Stop transfusion , supportive care
Transfusion associated circulatory overload
Excessive rate of transfusion
More likely in pre existing heart failure
Pulmonary oedmea and hypertension
Slow or stop transfusion. Consider IV loop diuretic
Transfusion-related acute lung injury
Hypoxia, pulmonary infiltrates on CXR, fever, hypotension
Stop transfusion. Oxygen and supportive care
Red blood cell transfusion thresholds and targets
Without ACS: threshold 70g/L
Target 70-90
With ACS: threshold 80 g/L
Target 80-100
Richter’s transformation presentation.
CLL transformation to a high grade non-Hodgkin lymphoma
Presents with rapidly growing lymph node, fever, weight loss, night sweats, nausea, abdo pain
CLL investigation findings
Leukocytosis
Anaemia
Thrombocytopenia
Smudge/smear cells on blood film
Immunophenotyping is done
Next steps if DVT likely on two level wells score
Ultrasound the leg - if DVT present start anticoagulation
If USS negative do a D-dimer
If negative DVT unlikely
If positive then re scan in a few days and monitor
If can’t USS within 4 hours then anticoagulate in the meantime
Next steps if DVT unlikely on two level wells score (1 point or less)
D dimer within 4 hours
- if positive then USS leg within 4 hours (anticoagulate if longer)
- if negative DVT unlikely
Choice of anticoagulant in DVT
DOACs from start in most patients (rivaroxaban or apixaban first line)
If not suitable LMWH followed by warfarin
E.g. in antiphospholipid syndrome or severe renal impairment
acute haemolytic reaction to blood products
ABO incompatibility - human error
fever, abdominal pain and hyptension
stop transfusion
Factor V Leiden
Activated protein C resistance
Most common form of inherited thrombophilia
G6PD deficiency
- summary of condition
- features
- diagnostic test
Most common red blood cell enzyme defect
Most common in people of Mediterranean and African descent
X linked
Can be precipitated by some drugs, infection, and broad/fava beans
Intravascular haemolysis
Neonatal jaundice
Gallstones common
Heinz bodies on film
Measure enzyme activity of G6PD
Hereditary spherocytosis
- summary
- features
- test
Autosomal dominant inheritance
More common in people of Northern European descent
Neonatal jaundice
Chronic symptoms of haemoylsis
Gallstones
Splenomegaly
Spherocytes on film
Diagnose with EMA binding test
Features of haemophilia
X linked
Haemarthroses
Haematoma
Prolonged bleeding after trauma or surgery
Prolonged APTT
Bleeding time, PT, thrombin time all normal
Immune thrombocytopenia in adults
Immune mediated reduction in the platelet count
More common in older females
May be detected on routine blood tests or present with petechiae, purpura, bleeding
Management is oral prednisone or IVIG if active bleeding
Iron studies in iron deficiency anaemia
Hb low
Ferritin low (but inflammation can raise it so high ferritin does not rule out IDA)
TIBC/transferrin high
Transferrin saturation low
Management IDA
Identify cause - may need further investigations e.g. endoscopy (new onset in older people always rule out malignancy)
oral ferrous sulphate until iron restored and then for 3 more months
Iron rich diet
Iron studies anaemia of chronic disease
Serum iron low but not as low as IDA
TIBC low
Transferrin saturation low
Ferritin high
What is a decrease in haptoglobin associated with
Intravascular haemolysis
Platelet transfusion risk
Highest risk of bacterial contamination
DVT or PE in pregnancy
Warfarin contraindicated
Give SC LMWH
Sideroblastic anaemia
Microcytic hypochromic anaemia
High iron ferritin and transferrin
Can be congenital or acquired
Causes of massive splenomegaly
Myelofibrosis
CML
Malaria
Gauchers syndrome
What is hydroxycobalamin
Vitamin B12 replacement
Given IM
Causes of neutropenia
Viral
Drugs e.g. cytotoxics, carbimazole
Benign ethnic neutropenia - common in black African or Afro Caribbean
Haematological malignancy
Rheumatological conditions
Severe sepsis
Correcting a mixed B12 and folate deficiency
Correct B12 first with IM injections then start oral folic acid once normal
